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Myelofibrosis

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https://www.readbyqxmd.com/read/28335073/clinical-characteristics-and-whole-exome-transcriptome-sequencing-of-coexisting-chronic-myeloid-leukemia-and-myelofibrosis
#1
Malathi Kandarpa, Yi-Mi Wu, Dan Robinson, Patrick William Burke, Arul M Chinnaiyan, Moshe Talpaz
Myeloproliferative neoplasms (MPNs) are clonal hematopoietic stem cell (HSC) disorders that can be classified on the basis of genetic, clinical, phenotypic features. Genetic lesions such as JAK2 mutations and BCR-ABL translocation are often mutually exclusive in MPN patients and lead to essential thrombocythemia, polycythemia vera or myelofibrosis (ET/PV/MF) or chronic myeloid leukemia, respectively. Nevertheless, coexistence of these genetic aberrations in the same patient has been reported. Whether these aberrations occur in the same stem cell or a different cell is unclear, but an unstable genome in the HSCs seems to be the common antecedent...
March 23, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28332891/the-positive-effect-of-deferasirox-on-erythropoiesis-in-a-patient-with-a-dual-diagnosis-of-myelofibrosis-and-diffuse-large-b-cell-lymphoma
#2
Louise Kostos, Khai Li Chai, Constantine S Tam, Ali Bazargan
No abstract text is available yet for this article.
March 23, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28324773/haptoglobin-is-frequently-low-in-patients-with-myelofibrosis-clinical-relevance
#3
Paolo Strati, Lucia Masarova, Prithviraj Bose, Naval Daver, Naveen Pemmaraju, Srdan Verstovsek
A recent study, showing the absence of paroxysmal nocturnal hemoglobinuria clones in myelofibrosis, has reopened the debate around the role of decreased haptoglobin in this disease. We present here a large prospective analysis of the clinical significance of low haptoglobin in 152 patients with myelofibrosis. Low haptoglobin (<32mg/dL) was observed in 50 patients (33%). Decreased haptoglobin did not associate with low hemoglobin levels, positive Coombs test or abnormal liver function tests, suggesting it is not result of autoimmune hemolytic anemia or liver cirrhosis...
March 8, 2017: Leukemia Research
https://www.readbyqxmd.com/read/28314843/quantitative-proteome-heterogeneity-in-myeloproliferative-neoplasm-subtypes-and-association-with-jak2-mutation-status
#4
Nuria Socoro-Yuste, Cokic Vladan P, Julie Mondet, Isabelle Plo, Pascal Mossuz
Apart from well-known genetic abnormalities, several studies have reported variations in protein expression in Philadelphia negative (Ph-) Myeloproliferative Neoplasm (MPN) patients that could contribute towards their clinical phenotype. In this context, a quantitative mass spectrometry proteomics protocol was used to identify differences in the granulocyte proteome with the goal to characterize the pathogenic role of aberrant protein expression in MPNs. LC MS/MS (LTQ Orbitrap) coupled to iTRAQ labeling showed significant and quantitative differences in protein content among various MPN subtypes [polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF)], and according to the genetic status of JAK2 (JAK2V617F presence and JAK2V617F allele burden)...
March 17, 2017: Molecular Cancer Research: MCR
https://www.readbyqxmd.com/read/28295973/cutaneous-phaeohyphomycosis-in-a-hematopoietic-stem-cell-transplant-patient-caused-by-alternaria-rosae-first-case-report
#5
Amy W Liu, Allen C Bateman, Adam Greenbaum, Kanishka Garvin, Jill Clarridge, Jonathan Grim
Alternaria species have been reported as a rare cause of fungal infection in organ and stem cell transplant recipients, but to date no reports have been published of infection in humans caused by Alternaria rosae. Here, we report cutaneous A. rosae infection in a 66-year-old farmer with a history of primary myelofibrosis who had undergone allogeneic unrelated donor hematopoietic stem cell transplantation. Forty-nine days post transplant, he presented with a nodule on the thumb with no findings suggestive of disseminated infection...
March 13, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/28294327/fibroblast-dynamics-as-an-in-vitro-screening-platform-for-anti-fibrotic-drugs-in-primary-myelofibrosis
#6
Ciprian Tomuleasa, Sonia Selicean, Grigore Gafencu, Bobe Petrushev, Laura Pop, Cristian Berce, Anca Jurj, Adrian Trifa, Ana-Maria Rosu, Sergiu Pasca, Lorand Magdo, Mihnea Zdrenghea, Delia Dima, Alina Tanase, Ioana Frinc, Anca Bojan, Ioana Berindan-Neagoe, Gabriel Ghiaur, Stefan O Ciurea
Although the cause for bone marrow fibrosis in patients with myelofibrosis remains controversial, it has been hypothesized that it is caused by extensive fibroblast proliferation under the influence of cytokines generated by the malignant megakaryocytes. Moreover, there is no known drug therapy which could reverse the process. We studied the fibroblasts in a novel system using the hanging drop method, evaluated whether the fibroblasts obtain from patients are part of the malignant clone of not and, using this system, we screen a large library of FDA-approved drugs to identify potential drugs candidates that might be useful in the treatment of this disease, specifically which would inhibit fibroblast proliferation and the development of bone marrow fibrosis...
March 11, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28293461/a-rare-cause-of-cytopenia-in-a-patient-with-systemic-lupus-erythematosus-autoimmune-myelofibrosis
#7
Döndü Üsküdar Cansu, Hava Üsküdar Teke, Cengiz Korkmaz
Hematological abnormalities are very common in the course of systemic lupus erythematosus (SLE). Myelofibrosis is a bone marrow disorder in which there is excessive fibrous tissue formation in the bone marrow. Various benign and malignant disorders can cause or be associated with a diffuse increase in the bone marrow reticular tissue. Some diseases such as infections, neoplasms, and autoimmune diseases may also induce bone marrow fibrosis (secondary myelofibrosis). Cytopenia from autoimmune myelofibrosis (AIMF) in SLE is a rare condition...
March 2017: European Journal of Rheumatology
https://www.readbyqxmd.com/read/28282034/prognostic-impact-of-bone-marrow-fibrosis-in-polycythemia-vera-validation-of-the-iwg-mrt-study-and-additional-observations
#8
D Barraco, S Cerquozzi, C A Hanson, R P Ketterling, A Pardanani, N Gangat, A Tefferi
In 2012, the International Working Group for Myeloproliferative Neoplasms (MPN) Research and Treatment (IWG-MRT) reported an associations between mild bone marrow (BM) fibrosis (⩾grade 1) in polycythemia vera (PV) and a lower incidence of thrombosis during the clinical course and a higher risk of fibrotic progression. The objective in the current study of 262 patients with PV was to validate these observations and also identify other risk factors for myelofibrosis-free survival (MFFS). About 127 (48%) patients displayed ⩾grade 1 reticulin fibrosis at the time of diagnosis; presenting clinical and laboratory features were not significantly different between patients with or without BM fibrosis...
March 10, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/28278711/heat-shock-protein-27-hsp27-hspb1-expression-is-increased-in-patients-with-primary-and-secondary-myelofibrosis-and-may-be-affecting-their-survival
#9
Marko Lucijanic, Ana Livun, Katarina Marija Tupek, Tajana Stoos-Veic, Gorana Aralica, Iva Gecek, Vlatko Pejsa, Rajko Kusec
No abstract text is available yet for this article.
February 28, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28277061/%C3%AE-granule-biogenesis-from-disease-to-discovery
#10
Chang Hua Chen, Richard W Lo, Denisa Urban, Fred G Pluthero, Walter H A Kahr
Platelets are critical to hemostasis and thrombosis. Upon detecting injury, platelets show a range of responses including the release of protein cargo from α-granules. This cargo is synthesized by platelet precursor megakaryocytes or endocytosed by megakaryocytes and/or platelets. Insights into α-granule biogenesis have come from studies of hereditary conditions where these granules are immature, deficient or absent. Studies of Arthrogryposis, Renal dysfunction, and Cholestasis (ARC) syndrome identified the first proteins essential to α-granule biogenesis: VPS33B and VPS16B...
March 2017: Platelets
https://www.readbyqxmd.com/read/28273188/-treatment-options-and-limitations-in-the-management-of-myelofibrosis
#11
Gábor Körösmezey, Gabriella Gyõri, Gábor Rudas, Hanna Eid, Zsolt Nagy, Judit Demeter
Primary myelofibrosis has the worst outcome among classical chronic myeloproliferative neoplasms. The past decade has brought numerous discoveries elucidating the role of proliferative mutations in disease pathogenesis. Mutations of the genes JAK2, MPL and CALR are present in about 90 percent of all primary myelofibrosis cases. The prognosis of myelofibrosis is considered heterogeneous, the expected survival of patients may range from one year to more than a decade based on several prognostic factors. Estimated survival can be assessed based on clinical prognostic scores...
March 8, 2017: Magyar Onkologia
https://www.readbyqxmd.com/read/28273187/-the-role-of-driver-and-subclonal-mutations-in-pathogenesis-of-primary-myelofibrosis
#12
Réka Mózes, Ambrus Gángó, Zsófia Boha, Judit Csomor, Csaba Bödör
Primary myelofibrosis (PMF) is a Philadelphia chromosome negative, clonal myeloproliferative neoplasm characterised by a progressive nature. Morphologically, the bone marrow biopsy shows features of abnormal proliferation of terminally differentiated megakaryocytes and subsequent bone marrow fibrosis. The molecular landscape of PMF includes phenotypic driver mutations (JAK2 V617F, CALR and MPL) which represent major diagnostic criteria, and subclonal mutations that also occur in several other myeloid diseases, but have a prognostic value in disease progression of MF...
March 8, 2017: Magyar Onkologia
https://www.readbyqxmd.com/read/28264600/quality-and-cost-comparison-of-powered-versus-manual-bone-marrow-biopsy-devices-in-patients-with-myelofibrosis
#13
Abhishek Maiti, Nicholas J Short, Srdan Verstovsek, Cynthia A Powers, Cheryl A Fullmer, Steven R Reyes, Carlos E Bueso-Ramos
No abstract text is available yet for this article.
February 28, 2017: Leukemia & Lymphoma
https://www.readbyqxmd.com/read/28263921/the-impact-of-splenectomy-in-myelofibrosis-patients-prior-to-allogeneic-hematopoietic-stem-cell-transplantation
#14
Marie Robin, Maryem Zine, Sylvie Chevret, Véronique Meignin, Nicolas Munoz-Bongrand, Hannah Moatti, Aliénor Xhaard, Flore Sicre de Fontbrune, Régis Peffault de Latour, Emile Sarfati, Gérard Socié
Performing a pre-transplant splenectomy in patients with myelofibrosis (MF) is a matter of debate, as while the procedure improves hematological recovery it may lead to severe morbidities. We retrospectively analyzed data from 85 patients consecutively transplanted in our center for MF, including 39 patients who underwent splenectomy before their transplant. A majority of them had primary MF (78%), were considered high risk patients (84% DIPSS intermediate-2 or higher), and were transplanted from HLA-matched sibling donors (56%) after a reduced-intensity conditioning regimen (82%)...
March 2, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28261298/fas-fasl-gene-polymorphisms-in-turkish-patients-with-chronic-myeloproliferative-disorders
#15
Fusun Gediz Ozdemirkiran, Sinem Nalbantoglu, Zafer Gokgoz, Bahriye Kadriye Payzin, Filiz Vural, Seckin Cagirgan, Afig Berdeli
INTRODUCTION: Chronic myeloproliferative disorders (CMPD) are chronic myeloid hematological disorders, characterized by increased myeloid cell proliferation and fibrosis. Impaired apoptotic mechanisms, increased cell proliferation, uncontrolled hematopoietic cell proliferation and myeloaccumulation may contribute to the pathogenesis of CMPD. The aim of our study was to show the possible role of FAS/FASL gene polymorphisms in CMPD pathogenesis and investigate the association with clinical parameters and susceptibility to disease...
March 1, 2017: Archives of Medical Science: AMS
https://www.readbyqxmd.com/read/28260257/a-case-based-discussion-of-clinical-problems-in-the-management-of-patients-treated-with-ruxolitinib-for-myelofibrosis
#16
P Joy Ho, Ashish Bajel, Kate Burbury, Lindsay Dunlop, Simon Durrant, Cecily Forsyth, Andrew C Perkins, David M Ross
Ruxolitinib is a dual janus kinase 1 (JAK1)/JAK2 inhibitor used to treat splenomegaly and symptoms associated with myelofibrosis (MF). Current therapeutic options for symptomatic MF include supportive care, myelosuppressive therapy (such as hydroxycarbamide) and janus kinase (JAK) inhibitors (in particular ruxolitinib). Allogeneic stem cell transplantation remains the only potentially curative treatment for MF, and younger transplant-eligible patients should still be considered for allogeneic stem cell transplantation; however, this is applicable only to a small proportion of patients...
March 2017: Internal Medicine Journal
https://www.readbyqxmd.com/read/28260027/pstat5-and-erk-exhibit-different-expression-in-myeloproliferative-neoplasms
#17
Ewa Wiśniewska-Chudy, Łukasz Szylberg, Grzegorz Dworacki, Ewa Mizera-Nyczak, Andrzej Marszałek
Myeloproliferative neoplasms (MPNs) are clonal hematopoietic progenitor cell disorders characterized by the proliferation of one or more hematopoietic lineages. The classical MPNs include polycythemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) entities. These disorders are characterized by bone marrow morphology typical for each disease, and by the presence of JAK2V617F mutation in the marrow and blood. However, JAK2V617F cannot account for the phenotypic heterogeneity of MPNs because approximately half of all cases of ET and PMF show no evidence of this molecular marker...
February 24, 2017: Oncology Reports
https://www.readbyqxmd.com/read/28259826/medical-management-of-extensive-arterial-thromboembolism-in-a-patient-with-essential-thrombocythemia-and-warfarin-failure
#18
Aisha K Ahmed, Alicia Youssef, Nedaa Skeik
Chronic myeloproliferative disorders (CMPD) share a stem cell-derived clonal myeloproliferation. This group of disorders includes essential thrombocythemia (ET), polycythemia vera (PV), chronic myeloid leukemia (CML), and primary myelofibrosis (PMF), with the respective features of thrombocytosis, erythrocytosis, and bone marrow fibrosis(1). These disorders can be associated with genetic mutations affecting protein tyrosine kinases, resulting in different configurations of abnormal signal transduction. The Janus tyrosine kinase 2 (JAK2) mutation can be used as a key diagnostic tool for diagnosing MPDs, specifically, ET, PV, and PMF(2)...
March 1, 2017: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/28248313/long-term-findings-from-comfort-ii-a-phase-3-study-of-ruxolitinib-vs-best-available-therapy-for-myelofibrosis
#19
C N Harrison, A M Vannucchi, J-J Kiladjian, H K Al-Ali, H Gisslinger, L Knoops, F Cervantes, M M Jones, K Sun, M McQuitty, V Stalbovskaya, P Gopalakrishna, T Barbui
No abstract text is available yet for this article.
March 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28247057/novel-hematological-parameters-for-the-evaluation-of-patients-with-myeloproliferative-neoplasms-the-immature-platelet-and-reticulocyte-fractions
#20
Paolo Strati, Prithviraj Bose, Lindsey Lyle, Katie Gaw, Lingsha Zhou, Sherry A Pierce, Julie Huynh-Lu, Cheryl F Hirsch-Ginsberg, Daniel E Bueso-Mendoza, Carlos E Bueso-Ramos, Srdan Verstovsek
New automated hematology analyzers have led to the availability of novel hematological parameters, including the immature platelet fraction (IPF) and the immature reticulocyte fraction (IRF), both of potential interest in patients with myeloproliferative neoplasms (MPNs). We performed a prospective analysis of 217 patients with MPN, including 32 (15%) with essential thrombocythemia (ET), 43 (20%) with polycythemia vera (PV), and 142 (65%) with myelofibrosis (MF); the IPF and IRF were measured by the Sysmex XN analyzer...
February 28, 2017: Annals of Hematology
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